Intracranial atypical teratoid/rhabdoid tumor:Pathological,clinical and imaging features / 医学研究生学报

ABSTRACT

Atypical teratoid/rhabdoid tumor of the central nervous system has aggressive growth with high potential CSF dissemination and frequently affects very young children.The tumor contains rhabdoid cells and primitive neuroectodermal,malignant mesenchymal and epithelial elements.Immunohistochemistry is helpful in differentiating atypical teratoid/rhabdoid tumor from primitive neuroectodermal tumor/medulloblastoma.Atypical teratoid/rhabdoid tumor is cytogenetically characterized by monosomy 22 or deletion of chromosome band 22q11.2.Intensified therapy combined with surgical resection has been suggested for its treatment.Due to the secondary cystic/necrotic changes and hemorrhage,the imaging features of the tumor are variable.